systemic sclerosis and raynauds Flashcards
scleroderma
skin thickening
which gender is it more common in
women
what do patients often present with in systemic sclerosis
raynauds
variations of systemic scleroiss
diffuse and limited
where does diffuse affect
above and below elbows and knees and face and trunk; whole body
what does limited affect
only below elbows and knees plus face
CREST of systemic sclerosis
CREST
calcinosis ; calcium deposits in skin
raynauds
esophageal dysfunciton; acid reflux
scleroderma; thickening of skin on hands and fingers
telangiectasis; dilation of capillaries causing red dots around face
causes of systemic sclerosis
genetic predisposition and environmental triggers
pathophysiology
result of vascular damage in the skin and organs
symptoms and signs
fatigue weakness
joint stiffness/pain
skin fibrosis presenting as thickened plaques
lack of wrinkles
tightenting of the skin around the mouth
unpleasant risk
pulmonary hypertension
what autoantibody is associated with pulmonary hypertension in limited ssc
anti centromere antibody - ACA
what autoantibody assoc with diffuse in progressive interstitial lung disease
anti topoisomerase SCL-7
non medical help
- Avoid smoking
- Gentle skin stretching to maintain the range of motion
- Regular emollients
- Avoiding cold triggers and wearing gloves for Raynaud’s
- Physiotherapy to maintain healthy joints
Occupational therapy for adaptations to daily living to cope with limitations
medical help for raynauds
oral vasodilators including ccbs and phosphodiesterase inhibitors
Iv vasodilators
immunomodulatory therapies for skin thickening
mycophenolate and methotrexate and cyclophosphamide
what is raynauds
hands become white in cold weather due to vasospasm and vasoconstriction
fingers become pure white
how do fingers become blue in raynauds
deoxygenated blood is floating around
what happens to raynauds fingers once back in heat
become red and stingy
primary raynauds
happens when young not really likely an auto immune disease
causes of secondary raynauds
scleroderma
rheumatoid arthritis
SLE
leukemia
use of vibrating tools
contraceptive pill
what is raynauds phenomenon
secondary raynauds
management of raynauds
ccb - nifedipine
IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months
